An Echinochloa rotundiflora in uska species han Liliopsida nga ginhulagway ni Clayton. An Echinochloa rotundiflora in nahilalakip ha genus nga Echinochloa, ngan familia nga Poaceae. Waray hini subspecies nga nakalista.

Mga kasarigan

Mga sumpay ha gawas

Echinochloa

Echinochloa rotundiflora


Viscount Thurso, of Ulbster in the County of Caithness, is a title in the Peerage of the United Kingdom. It was created on 11 June 1952 for the Scottish Liberal politician and former Secretary of State for Air, Sir Archibald Sinclair, 4th Baronet. His son, the second Viscount, served as Lord Lieutenant of Caithness from 1973 to 1995.

 the titles are held by the latter's son, the third Viscount, who succeeded in 1995. Known as John Thurso, he is a Liberal Democrat politician. Thurso lost his seat in the House of Lords after the passing of the House of Lords Act 1999, but was elected to the House of Commons in 2001, thereby becoming the first hereditary peer of the United Kingdom allowed to sit in the Commons without first disclaiming his title; he held his seat until his defeat in the 2015 general election. On 19 April 2016, he re-entered the Lords following his election by the remaining Liberal Democrat hereditary peers after the death of Lord Avebury. The third Viscount also became Lord Lieutenant of Caithness in 2017.

The Sinclair baronetcy, of Ulbster in the County of Caithness, was created in the Baronetage of Great Britain in 14 February 1786 for the first Viscount's great-great-grandfather, the Scottish politician and writer John Sinclair. Both his son, the second Baronet, and grandson, the third Baronet, represented Caithness in the House of Commons. The latter was the grandfather of the fourth Baronet, who was elevated to the peerage as Viscount Thurso in 1952.

The family seats are Orchard Cottage, near Champneys, Hertfordshire, and Thurso Castle in the Scottish Highlands.

Sinclair baronets, of Ulbster (1786)
Sir John Sinclair, 1st Baronet (1754–1835)
Sir George Sinclair, 2nd Baronet (1790–1868)
Sir John George Tollemache Sinclair, 3rd Baronet (1824–1912)
Clarence Granville Sinclair (1858–1895)
Sir Archibald Henry MacDonald Sinclair, 4th Baronet (1890–1970) (created Viscount Thurso in 1952)

Viscounts Thurso (1952)
Archibald Henry Macdonald Sinclair, 1st Viscount Thurso (1890–1970)
Robin Macdonald Sinclair, 2nd Viscount Thurso (1922–1995)
John Archibald Sinclair, 3rd Viscount Thurso (born 1953)

The heir apparent is the present holder's son, the Hon. James Alexander Robin Sinclair (born 1984).

Male-line family tree

Line of succession

 Rt. Hon. Sir John Sinclair, 1st Baronet (1754–1835)
 Sir George Sinclair, 2nd Baronet (1790–1868)
 Sir John George Tollemache Sinclair, 3rd Baronet (1824–1912)
 Lt Col. Clarence Granville Sinclair (1858–1895)
  Archibald Henry Macdonald Sinclair, 1st Viscount Thurso (1890–1970)
  Robin Macdonald Sinclair, 2nd Viscount Thurso (1922–1995)
  John Archibald Sinclair, 3rd Viscount Thurso (born 1953)
 (1) Hon. James Alexander Robin Sinclair (born 1984)
 (2) Hon. George Henry MacDonald Sinclair (born 1989)
 (3) Hon. Patrick James Sinclair (born 1954)
 (4) Jody Peter Fergus North Sinclair (born 1975)
 (5) Luke Robin Sinclair (born 1979)
 Rev. William Sinclair (1804–1878)
 Ven. John Stewart Sinclair (1853–1919)
 Sir John Alexander Sinclair (1897–1977)
 (6) Ian Alexander Sinclair (born 1938)
 (7) Andrew George Sinclair (born 1970)
 (8) John Charles Sinclair (born 1972)
 (9) Roderick John Sinclair (born 1944)
 (10) James Alexander Sinclair (born 1984)

Only those in positions (1) to (5) are in remainder to the viscountcy. Those in positions (6) to (10) are in remainder to the baronetcy only.

See also
Veronica Linklater, Baroness Linklater of Butterstone

Notes

References
Kidd, Charles, Williamson, David (editors). Debrett's Peerage and Baronetage (1990 edition). New York: St Martin's Press, 1990,

External links

www.debretts.co.uk

Viscountcies in the Peerage of the United Kingdom
Noble titles created in 1952
Noble titles created for UK MPs

Viscount Thurso


El carpintero panameño o carpintero carirrayado (Piculus callopterus) es una especie de ave de la familia Picidae, endémico de Panamá.

Hábitat
Vive en el nivel bajo y medio del bosque húmedo y en los bordes de éste, principalmente en colinas, entre los 300 y 900 m de altitud.

Descripción
Mide aproximadamente 18 cm de longitud. Los machos son de color marrón anaranjado arriba, con una grupa amarillenta con rayas oliva, pecho y garganta de color oliva con manchas blancas y las partes inferiores color amarillo opaco. Su corona, nuca y frente son de color rojo brillante y, tiene una raya blanca en la mejilla inferior. Las hembras son similares a los machos, pero tienen corona y frente de color gris oscuro y solamente la nuca roja.

Taxonomía
Anteriormente era considerado como subespecie de Piculus leucolaemus, pero por las diferencias en los patrones faciales y la voz y además , por el hecho de que no se cruzan nunca en las áreas en las cuales se superponen, son consideradas ahora como especies diferentes por la American Ornithologists' Union.

Referencias

callopterus
Aves de América Central
Taxones descritos por George Newbold Lawrence

Piculus callopterus


Kompenzator (njem. Kompensator od engl. compensator, prema lat. compensare: ujednačiti, naknaditi) može značiti:
 kompenzator, općenito, je ono (ili onaj) što teži uspostaviti funkcionalnu ravnotežu.
 kompenzator (optika), u optici, je leća ili sustav leća, prizmi, dijafragmi i slično kojima se ispravljaju pogreške u stvaranju slike (na primjer kromatska i sferna aberacija).
 kompenzator (elektrotehnika), u elektrotehnici, je sinkroni električni stroj, posebno građen trofazni sinkroni generator koji služi za kompenzaciju jalove energije potrebne za rad mnogih elektrotehničkih naprava, uređaja i strojeva poput transformatora, asinkronih motora, prigušnica, ispravljača, lučnih peći, fluorescentnih svjetiljki. Jalova induktivna i kapacitivna energija stvarana u sinkronom kompenzatoru omogućuje i regulaciju električnog napona u elektroenergetskoj mreži. Za kompenzaciju jalove energije u industrijskim i distributivnim postrojenjima sve se više koriste električni kondenzatori.
 kompenzator (strojarstvo), u strojarstvu, je strojni dio mehaničkih konstrukcija, postrojenja ili strojeva kojim se smanjuju ili poništavaju sile koje nastaju toplinskim stezanjem ili rastezanjem (dilatiranjem) njihovih dijelova kao posljedice promjene temperature ili slično. Na mjestu ugradbe kompenzator omogućuje međusobne pomake toplinski rastezanih dijelova zahvaljujući vlastitoj elastičnosti ili izvedbi u obliku kliznoga spoja. Gdjekad se cijeli dio postrojenja izvodi tako da dopušta povećane pomake, na primjer kod cjevovoda s pomoću takozvanih dilatacijskih lira u obliku velikoga grčkog slova omega.  Dilatacija (razdvojba)

Izvori

Kompenzator


Kathrine Windfeld er navnet på flere personer:

 Kathrine Windfeld (filminstruktør) - en dansk filminstruktør (1966-2015)
 Kathrine Windfeld (musiker) - en dansk jazzpianist mv. (født 1984)

Kathrine Windfeld


Metacafe, Temmuz 2003'te kurulan İsrailli bir video paylaşım sitesiydi. Ağustos 2021'de platformun web sitesi pasif hale geldi ve sosyal medya sayfaları da terk edildi.

Tarihi ve Kullanımı
Metacafe şirketi 2003'te  Eyal Herzog (CTO), Arik Czerniak (CEO) ve Ofer Adler (Board Member) tarafından, özel yatırımlar ve kendi paralarıyla toplam 4 milyon dolar fon ile kurulmuştur.

2006'daki kuruluşundan itibaren site trafiği çok hızlı arttı. Alexa'ya göre 128. sıraya kadar yükseldi.

Temmuz 2006'da, ikinci mali döneme girildiğinde bütçesi 15 milyon dolara kadar yükseldi.

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 Windows kullanarak -Bedava indirilebilir bir program ile kullanıcılar kendi videolarını geliştirme ve bu siteye yüklemeye kâdir olurlar. Bu program dosyaları kendi internetine göre indirir veya siteye yükler.

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Metacafe'nin Japonya'da ve İsrail'de binası bulunmaktaydı.

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Metacafe'nin porno karşıtı politikası vardı. Fakat çizgi filmsel içeriğe izin vermekteydi. Normal olarak bu filtre kullanılırdı. Aile filtresine rağmen, birçok Metacafe kullanıcısı birçok videoda kırıcı yorumlar yapmaktaydı. Bunlar seks, ırk, ve bunun benzeri kötü yorumlardı. Bunların kaldırılması en az 3 kişinin şikayetiyle olanaklıydı.

Türkiye'de erişimin engellenmesi
9 Mayıs 2010 tarihinde Deniz Baykal'a yönelik komplo kaset videoları yayınlandığı için mahkeme kararıyla engellenmiştir.

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Kurt Browning,  (born June 18, 1966) is a Canadian figure skater, choreographer and commentator. He is the first skater to land a ratified quadruple jump in competition. He is a four-time World Champion and Canadian national champion.

Career
Browning was both Canadian figure skating champion and World Champion four times. He represented Canada in three Winter Olympics, 1988 (finishing 8th overall), 1992 (6th) and 1994 (5th), and earned the privilege of carrying the Canadian flag during the opening ceremonies of the 1994 games in Lillehammer, Norway. Browning's other achievements include three Canadian Professional Championships and three World Professional Championships.

On March 25, 1988, at the 1988 World Championships in Budapest, Hungary, Browning landed the first ratified quadruple jump, a toe loop in the competition. This accomplishment is listed in the Guinness Book of Records. Jozef Sabovčík had previously landed a quad toe loop at the 1986 European Championships which was recognized at the event but then ruled invalid three weeks later. Browning later said, "I remember that there were a few people landing the jump (in practice) long before I did, and by watching them I was inspired to try it myself. After landing it, I certainly expected more skaters to start doing it in competition. I was surprised in the next few years when that really did not happen." Browning is also known for his intricate, fast, often lighthearted footwork. Browning has used percussive pop instrumentals in his programs; for example, he used "Bonzo's Montreux" by Led Zeppelin in 1993. He interpreted the music by punching his fists and frequently running across the ice. The following season he used "St. Louis Blues," wearing a tight black t-shirt, stretch pants, and belt. He also raised his fists "in a biceps-flexing muscle pose" and included humor by emulating laughter with upper body movements, skated in time to descending saxophone notes and by pointing to the audience during his footwork sequence.

One of Browning's iconic programs is "Singin' in the Rain", where he emulates Gene Kelly's dancing in the film. Choreographed by Sandra Bezic and debuted in a CBC-TV special in 1994, this program is still asked to be performed at ice shows after 20 years.

As a commentator, Browning has made frequent appearances since turning professional in 1994. He has been a regular colour commentator for the CBC at major skating events since 2006.

Browning appeared in the 2006 Fox television program Skating with Celebrities. Starting in 2009, he was the co-host of Battle of the Blades.

As a choreographer, he has choreographed programs for many figure skaters including:

 Jeremy Abbott
 Kevin Alves
 Patrick Chan
 Vaughn Chipeur
 Marc-André Craig
 Alissa Czisny
 Javier Fernández
 Yuzuru Hanyu
 Takeshi Honda
 Brian Joubert
 Tuğba Karademir
 Carolina Kostner
 Takahiko Kozuka
 Tara Lipinski
 Evan Lysacek
 Brandon Mroz
 Lucinda Ruh
 Jamie Salé / David Pelletier
 Yuka Sato
 Tomáš Verner
 Yan Han

He also co-directed and choreographed the 2011–12 Stars on Ice tour.

Awards and honors
Browning was awarded with a Lou Marsh Trophy for top Canadian Athlete (in 1990), Lionel Conacher Awards (1990 and 1991), Order of Canada (in 1990), an American Skating World Professional Skater of the Year Award (in 1998), and a Gustav Lussi Award from the Professional Skaters' Association (in 2001).

Browning was inducted into Canada's Sports Hall of Fame in 1994 and Skate Canada Hall of Fame in 2000. He was honored with a star on Canada's Walk of Fame in 2001. He was inducted into World Figure Skating Hall of Fame in 2006. He received the inaugural ISU Skating Lifetime Achievement Award in 2020.

Personal life
Browning was born on June 18, 1966, in Rocky Mountain House, Alberta. He was raised in Caroline, Alberta.

He married Sonia Rodriguez, a principal dancer with the National Ballet of Canada, on June 30, 1996. Their first son, Gabriel, was born on July 12, 2003, and their second son, Dillon, was born on August 14, 2007. They are now divorced.

Browning's home in the Forest Hill area of Toronto suffered a fire on August 18, 2010.

He married Alissa Czisny on August 11, 2022.

Competitive highlights

References

External links

 Kurt Files

1966 births
Sportspeople from Alberta
Canadian male single skaters
Northern Star Award winners
Olympic figure skaters for Canada
Figure skaters at the 1988 Winter Olympics
Figure skaters at the 1992 Winter Olympics
Figure skaters at the 1994 Winter Olympics
Living people
Members of the Order of Canada
People from Clearwater County, Alberta
Figure skating commentators
World Figure Skating Championships medalists
Canadian television hosts
Goodwill Games medalists in figure skating
Competitors at the 1990 Goodwill Games

Kurt Browning


الخط المغربي يشير إلى مجموعة الخطوط العربية المترابطة التي تطورت في بلاد المغرب (شمال إفريقيا) والأندلس (شبه الجزيرة الإيبيربية) وغرب بلاد السودان (منطقة الساحل). ينحدر الخط المغربي من الخط الكوفي، ويكتب بقلم سميك، ليبدو الخط في غاية الاتزان، ويستعمل في أعمال النساخة والتدوين والزخرفة.

تاريخ

ظهور
دخلت الكتابة العربية إلى بلاد المغرب منذ القرن الأول هـ/7م أي مع الفتح الإسلامي للمغرب، وقد وقع تبني كل ما ورد من المشرق، من أساليب كتابية ومن بينها الخط الكوفي، حيث ورد على مدينة القيروان عاصمة المنطقة ومنها انتشر إلى بقية أرجاء بلاد المغرب. ينحدر الخط المغربي من الخط الكوفي مباشرة، فاشتق من الخطوط المشرقية قبل تطبيق إصلاحات الخط التي وضعها ابن مقلة فيما يعرف بالخط المنسوب.

وفي القيروان ما لبث الخط الكوفي أن تطور ليتخذ أساليب وخصائص جديدة حتى تسمى بالخط القيرواني الذي كان يستعمل لكتابة المصاحف، كما تطور خط نسب إلى مدينة المهدية، عاصمة الفاطميين.

انتشار
انتشر الخط المغربي من العواصم مثل القيروان وفاس وقرطبة. وكان الخط المغربي يستعمل خلال قرون في كتابة المخطوطات العربية التي تداولت في بلاد المغرب. ووفق المؤرخ المغربي محمد المنوني كان هناك 104 مصنع ورق في فاس تحت حكم يوسف بن تاشفين في عهد المرابطين، و400 مصنع ورق تحت حكم السلطان يعقوب المنصور في عهد الموحدين.

فكان كلما دخل منطقة سمي باسمها، وعلى هذا المنوال جاءت تَسمية هذا الخط بالخط القيرواني، ولما دخل إلى المغرب سمي بالخط المغربي وهنا بدء الإبداع فيه وتجويد أنواعه وسمي الخط الفاسي والخط المراكشي.

أما بالأندلس، فقد تطور فيها الخط الكوفي أيضًا، وظهر نوعان أساسيان تكثر في أحدهما الزوايا سمي بالكوفي الأندلسي، وتكثر في الآخر الانحناءات والاستدارات سمي بالقرطبي أو الأندلسي، وقد استخدم في نسخ المصاحف والكتب.

استمر التمايز بين خطوط الأندلس وشمال إفريقيا إلى القرن الرابع هـ/10م. ثم خضعت شمال إفريقيا للتأثير الأندلسي إثر قدوم المهاجرين الأندلسيين إليها في موجات، مع بدء تراجع النفوذ العربي الإسلامي بالأندلس. وفي هذا المعنى يقول ابن خلدون: أما أهل الأندلس فانتشروا في الأقطار منذ تلاشي ملك العرب ومن خلفهم من البربر... فانتشروا في عدوة المغرب وإفريقية... وتعلقوا بأذيال الدولة، فغلب خطهم على الخط الإفريقي وعفا عليه، ونسي خط القيروان والمهدية... وصارت خطوط أهل إفريقية كلها على الرسم الأندلسي بتونس وما إليها... وبقي منه رسم ببلاد الجريد الذين لم يخالطوا كتاب الأندلس... وحصل في دولة بني مرين من بعد ذلك بالمغرب الأقصى لون من الخط الأندلسي لقرب جوارهم وسقوط من خرج منهم إلى فاس قريبا واستعمالهم إياهم سائر الدولة ونسي عهد الخط فيما بعد عن سدة الملك وداره كأنه لم يعرف. وهو ما يعني أن الخط الأندلسي حل محل الخطوط المحلية المتأثرة بالقيروان، وقد استثنى ابن خلدون منطقة بلاد الجريد لأنها لم تتصل بالأندلسيين. وتطور الخط الأندلسي بدوره ومنه تولد ما عرف فيما بعد بالخط المغربي.

عرف الخط المغربي في بلاد المغرب الأقصى تدهورا تزامن مع فترة التدهور هناك في عهد السلطان العلوي محمد الثالث، وسمي هذا الخط غير المقروء بالخط البدوي.

بادر بعض الخطاطين المغاربة إلى الكتابة في موضوع الخط وقواعده، لضبط أساليبه وأشكاله، ووضعها رهن إشارة مريدي الخط المغربي مثل منظومة "نظم لآلي السمط في تحسين بديع الخط" وشرحها "حلية الكاتب ومنية الطالب" للخطاط أحمد بن قاسم الرفاعي الرباطي (1841م).

أصبح الخط المغربي المجوهر الخط المعتمد في الطباعة الحجرية بعد أن جلب محمد ابن الطيب الروداني من مصر أول مطبعة حجرية في المغرب الأقصى عام 1864.

من ضمن منشورات المستشرق الفرنسي أوكتاف هودا عن الخط المغربي هناكEssai sur l'Ecriture Maghrebine عام 1886 و Recueil de Lettres Arabes Manuscrites عام 1891.

أنواعه

يذكر خمسة أنواع فرعية للخط المغربي في كتاب الخط المغربي: تاريخ وواقع وآفاق:

 الكوفي المغربي، أسلوب في الخط الكوفي ابتكر في بلاد المغرب والأندلس
 الكوفي المرابطي، نوع من الكوفي المغربي اعتمدته الدولة المرابطية
 المبسوط، خط اعتيادي مناسب لفقرات النص وللقرآن. يعتبر امتداد للخط الكوفي وعرف تجويدا في المغرب وفي الأندلس. اعتمدته المطبعة الحجرية بفاس لطباعة المصحف القرآني. يشابه خط النسخ في الاستعمال.
 المجوهر، يستعمله الملك في إعلان الظهائر
 الثلث المغربي، وكان يسمى الخط المشرقي أو الخط المشرقي المتمغرب، هو مستوحى من خط الثلث المشرقي
 المسند، ويسمى كذلك الزمامي، هو خط يستعمله العدول والمحاكم في كتابة عقود الزواج وما إلى ذلك. تطور من الخط المغربي المجوهر والحروف في هذا الخط تميل إلى اليمين. وهذا الخط كان يستعمل في النصوص التي يريد المحررون إضفاء طابع من الغموض عليها، مثل النصوص المرتبطة بالسحر، وذلك نظرا لصعوبة قرائته.

وإلى ذلك هناك الخط القندوسي، وهو خط مغربي مبدع ابتكره الخطاط المتصوف محمد بن القاسم القندوسي في القرن التاسع عشر.

وكان المستشرق الفرنسي أكتاف هودا يصف أربعة أنواع من الخط المغربي وينسبها إلى مدن أو بلدان معينة:

 القيرواني: يمتاز بحروفه القصيرة والقريبة من بعضها؛
 الأندلسي: الخطوط التي ابتكرت بالأندلس
 الخط الفاسي: ويمتاز بطول الأسطر العمودية؛
 السوداني أو التمبكتي : نسبة إلى بلاد السودان (أي إفريقيا جنوب الصحراء) أو مدينة تمبكتو (بمالي الحالي)، ويمتاز بكبره وغلظه؛

الخط السوداني
هناك خطوط عربية من غرب إفريقيا تصنف ضمن الخطوط المغربية ويشار إليها باسم الخط السوداني، من بلاد السودان، ومن أنواع الخط السوداني هناك:

 الخط السوقي نسبة إلى مدينة السوق، واستعمل كذلك في تمبكتو ويرتبط بالطوارق.
 الخط الفولاني
 الخط الهاوساوي
 الخط البيضاني الموريطاني
 الخط الكنيمي أو الكنوي يرتبط بالمنطقة التي هي تشاد وشمال نيجيريا الآن، ويرتبط.
 الخط الصحراوي

بعض خصائصه

 ليس للخط المغربي قواعد محددة أو موازين كما هو الحال بالنسبة للخطوط العربية الأخرى. ولانعدام تلك القواعد فإن الحرف الواحد قد يكتب بأكثر من طريقة في نفس الوثيقة.
 يحتفظ الخط المغربي ببعض الرواسب التي ورثها من الخط الكوفي من ذلك أن الألف المتصلة تنحدر قليلا عن مستوى السطر بزائدة كوفية، كما أن حروف الكاف والصاد والضاد والطاء والظاء تكون ممتدة بما يذكر بالخط الكوفي.
 قلما تحتفظ حروف الألف واللام والطاء والظاء بشكلها العمودي إذ كثيرا ما تكون في شكل منحني وتحمل في أعلاها ما يشبه النقطة الغليظة. وبالنسبة لحرف الظاء قد ترسم النقطة على يسار الشلة.
 لا ترسم عادة السنينة بعد حرفي الصاد والضاد، وقد ترسم نقطة الضاد داخل الحرف.
 تتخذ بعض الحروف امتدادا مبالغا فيه إذا كانت في آخر الكلمة وأساسا منها س ش ي ل ن.
 كما قد لا تكتب نقط الحروف النهائية في الفاء والقاف والنون.
 يتميز الخط المغربي فيما يتعلق بإعجام حرفي الفاء والقاف، حيث ترسم الفاء بنقطة من أسفل والقاف بنقطة واحدة من فوق.

مخطوطات قرآنية بخط مغربي
نسخة مغربية مزوقة لمصحف تمت كتابتها في 1148 هجري، من مجموعة شالوم يهودا، المكتبة الوطنية.

المراجع
 ابن خلدون، المقدمة، ص 507-508.
 - الأمين، إيمان، الخط المغربي : أصوله، مراحل تطوره ومدارسه، رسالة ختم الدروس الجامعية، المعهد الأعلى للتوثيق 1999-2000.
 المنوني، محمد، تاريخ الوراقة المغربية، صناعة المخطوط المغربي من العصر الوسيط إلى الفترة المعاصرة، منشورات كلية الآداب والعلوم الإنسانية، الرباط، 1991.
 عبد اللطيف، محمد الصادق، «الخط المغربي.. الخصائص والأنواع» ، مجلة الكويت عدد 191، ص 107-109.
 عبد اللطيف، محمد الصادق، «جماليات الخط المغربي» ، مجلة القافلة (الظهران-السعودية)، رمضان 1415هـ/فيفري 1995، ص 9-12.
 المنوني، محمد، «لمحة عن تاريخ الخط العربي والزخرفة في الغرب الإسلامي» ، في، المجلة التاريخية المغربية، ع 53-54، جويلية 1989، ص 205-230.
 هوداس، أُ، «محاولة في الخط العربي» ، تعريب عبد المجيد التركي، حوليات الجامعة التونسية، ع 3، 1966، ص 175-214.

انظتةر أيضا
 خط كوفي.
 خط الثلث.
 خط النسخ.

وصلات خارجية
  دراسة حول الخط المغربي.

مراجع

ثقافة تونسية
ثقافة جزائرية
ثقافة مالية
ثقافة مغربية
حضارة إسلامية
خطوط عربية
لهجات مغاربية

خط مغربي


Angelo Grimaldi ist ein italienischer Fernsehregisseur.

Grimaldi war neben seiner Arbeit für das italienische Fernsehen und als Agent zahlreicher Schauspieler mit einem Kompilationsfilm über die letzten Tage des Regimes Mussolini in den Kinos seines Heimatlandes vertreten. La repubblica di Mussolini R.S.I. entstand 1975.

Weblinks

Einzelnachweise

Fernsehregisseur
Italiener
Geboren im 20. Jahrhundert
Mann

Angelo Grimaldi


La partida de ajedrez es una pequeña pintura al óleo sobre tabla del pintor renacentista holandés Lucas van Leyden, que para entonces tenía catorce años. La obra representa una partida de ajedrez, en una variante originada en Alemania en el  popular allí y en los Países Bajos hasta principios del , entre una dama y un oponente, rodeados por varios observadores, entre los cuales otra dama con una toca blanca. Por entonces, esta variante del ajedrez, el kurierspiel, servía como una metáfora del cortejo amoroso. El cuadro se considera una de las primeras muestras de la pintura de género holandesa.

La joven sentada a la derecha juega con las negras asesorada por un hombre, probablemente su padre. Frente a ella, su anciano potencial pretendiente juega con las figuras blancas, entrecerrando los ojos mientras se echa hacia atrás. Un análisis moderno de la posición de las piezas llegó a la conclusión de que las negras están ganando, especialmente por que la joven está a punto de dar jaque a su oponente con una torre.

El cuadro ha sido reproducido con bastante frecuencia en obras sobre la historia del ajedrez y se encuentra actualmente en la Gemäldegalerie de Berlín.

Referencias

Enlaces externos

Colecciones de la Gemäldegalerie de Berlín
Cuadros de 1509
Escenas de género

La partida de ajedrez


Die Swedish Open 1983 im Badminton fanden vom 10. bis zum 13. März 1983 in Malmö, Schweden, statt. Es war das weltweit erste Turnier, das innerhalb der neu installierten Grand-Prix-Serie ausgetragen wurde. Das Turnier war in Kategorie 3 eingestuft.

Finalresultate

Referenzen
 http://newspapers.nl.sg/Digitised/Page/straitstimes19830315.1.39.aspx
 https://badmintonmuseet.dk/wp-content/uploads/2019/07/1983_04.pdf

World Badminton Grand Prix 1983
1983
Badmintonwettbewerb in Malmö

Swedish Open 1983


Kamenný Most este o comună slovacă, aflată în districtul Nové Zámky din regiunea Nitra. Localitatea se află la , se întinde pe o suprafață de  și în 2017 număra 1.058 de locuitori.

Istoric
Localitatea Kamenný Most este atestată documentar din 1271.

Note

Legături externe

Localități din Slovacia

Kamenný Most, Nové Zámky


Jacques Chaban-Delmas (; 7 March 1915 – 10 November 2000) was a French Gaullist politician. He served as Prime Minister under Georges Pompidou from 1969 to 1972. He was the Mayor of Bordeaux from 1947 to 1995 and a deputy for the Gironde département between 1946 and 1997.

Biography
Jacques Chaban-Delmas was born Jacques Michel Pierre Delmas in Paris. He studied at the Lycée Lakanal in Sceaux, before attending the École Libre des Sciences Politiques ("Sciences Po"). In the resistance underground, his final nom de guerre was Chaban; after World War II, he formally changed his name to Chaban-Delmas. As a general of brigade in the resistance, he took part in the Parisian insurrection of August 1944, with general de Gaulle. He was the youngest French general since François Séverin Marceau-Desgraviers, during the First French Empire.

A member of the Radical Party, he finally joined the Gaullist Rally of the French People (RPF), which opposed the Fourth Republic's governments. In 1947, he became mayor of Bordeaux, which was for 48 years his electoral fief. As a member of the National Assembly, he sat with the RPF.

In 1953, when the RPF group split (and Charles de Gaulle supposedly retired), Chaban-Delmas became head of the Union of Republicans for Social Action and president of the National Centre of Social Republicans party. He "tied up" with centre-left parties and joined Pierre Mendès-France's cabinet one year later as Minister of Public Works. He took part in the centre-left coalition Republican Front, which won the 1956 legislative election. He was France's Defence Minister in 1957–1958. His governmental participation during the Fourth Republic inspired the distrust of de Gaulle and some Gaullists.

Following Gen. de Gaulle's return to power in 1958, Chaban-Delmas agreed to the advent of the French Fifth Republic and the new Constitution. He took part in the foundation of the Union for the New Republic (UNR) and was elected, against de Gaulle's will, chairman of the National Assembly. He kept this function until the end of de Gaulle's presidency in 1969. Unlike some Gaullists, for instance, Jacques Soustelle, he supported de Gaulle's policy to end the Algerian War of Independence. During the 1959 UNR Congress, he was the first politician to evoke a "reserved presidential domain," composed chiefly of defence and diplomacy. This interpretation of the Constitution of 1958 has survived.

In 1969, when Georges Pompidou acceded to the presidency, he chose Chaban-Delmas, who had concluded that the May 68 crisis was the consequence of a strained and conflicted society, as prime minister. Chaban-Delmas tried to promote what he called "a new society", based on dialogue between the different social forces in French society. Amongst other reforms, government authority over the mass media was relaxed, while legislation was passed on social welfare coverage for the poor and elderly which consolidated France's profile as a welfare state. In addition, regular increases were made to the minimum wage which prevented greater wage disparities. A new legal aid scheme was introduced, along with a number of new social welfare benefits.

As a result of his social policies, Chaban-Delmas was viewed as too "progressive" by the "conservative" wing of the Gaullist movement. He was suspected of wanting to "tie up" again with the centre-left. Indeed, his advisers who inspired the "new society" programme were considered as close to the centre-left (Simon Nora and Jacques Delors who would serve as Finance Minister under François Mitterrand). Besides, a latent conflict opposed Chaban-Delmas to President Pompidou and the presidential circle. They accused him of trying to weaken the presidency in favour of himself. The satirical paper Le Canard Enchaîné accused him of breaking the law through tax evasion and in 1972, Chaban-Delmas canvassed for a vote of confidence in the Assembly. He did obtain this, but the President still managed to force his resignation.

Two years later, following the death in office of President Pompidou, Chaban-Delmas ran for the presidency himself. He was supported by the "lords of gaullism", but 43 personalities close to the late president, led by Jacques Chirac, published the Call of the 43 in favour of the candidacy of Valéry Giscard d'Estaing. Chaban-Delmas was defeated on the first ballot of the 1974 presidential election, winning only 15.10% of the vote. Chirac became President Giscard d'Estaing's prime minister.

Chaban-Delmas stood in the Gaullist Party (RPR) and, in spite of Chirac's leadership, returned to the chair of the National Assembly (1978–1981). Due to his friendship with President Mitterrand, his name was mentioned as a possible prime minister during the first "cohabitation" (1986–1988), but he instead became president of the National Assembly for the third time and Chirac again became premier.

Chaban-Delmas retired in 1997, towards the end of his thirteenth term of member of the National Assembly and two years after the end of his eighth term as Mayor of Bordeaux.

Political career

Governmental functions

Prime minister: 1969–1972

Minister of Public Works, Transport, and Tourism: June–August 1954 / 1954–1955

Minister of Housing and Reconstruction: September–November 1954

Minister of State: 1956–1957

Minister of Defence and Armed Forces: 1957–1958

Electoral mandates

National Assembly of France

President of the National Assembly: 1958–1969 / 1978–1981 / 1986–1988

Member of the National Assembly for the Gironde 2nd : 1946–1969 (Became Prime minister in 1969) / 1972–1997. Elected in June 1946, reelected in November 1946, 1951, 1956, 1958, 1962, 1967, 1968, 1973, 1978, 1981, 1988, 1993.

Regional Council

President of the Regional Council of Aquitaine : 1974–1979 / 1985–1988 (Resignation). Elected in 1986.

Regional councillor of Aquitaine : 1974–1979 / 1985–1988 (Resignation). Elected in 1986.

Municipal Council

Mayor of Bordeaux : 1947–1995. Reelected in 1953, 1959, 1965, 1971, 1977, 1983, 1989.

Municipal councillor of Bordeaux : 1947–1995. Reelected in 1953, 1959, 1965, 1971, 1977, 1983, 1989.

Urban Community Council

President of the Urban Community of Bordeaux : 1967–1983 / 1983–1995. Reelected in 1971, 1983, 1989.

Vice-president of the Urban Community of Bordeaux : 1977–1983.

Chaban-Delmas Cabinet

Minister of Foreign Affairs – Maurice Schumann
 ... National Defence – Michel Debré
 ... the Interior – Raymond Marcellin
 ... Economy and Finance – Valéry Giscard d'Estaing
 ... Industrial and Scientific Development – François-Xavier Ortoli
 ... Labour, Employment, and Population – Joseph Fontanet
 ... Justice – René Pleven
 ... National Education – Olivier Guichard
 ... Veterans and War Victims – Henri Duvillard
 ... Cultural Affairs – Edmond Michelet
 ... Agriculture – Jacques Duhamel
 ... Housing and Equipment – Albin Chalandon
 ... Transport – Raymond Mondon
 ... Relations with Parliament – Roger Frey
 ... Public Health and Social Security – Robert Boulin
 ... Posts and Telecommunications – Robert Galley

Changes
 19 October 1970 – André Bettencourt succeeds Michelet (d. 9 October 1970) as interim Minister of Cultural Affairs
 7 January 1971 – Jacques Duhamel succeeds Bettencourt as Minister of Cultural Affairs. Michel Cointat succeeds Duhamel as Minister of Agriculture. Jean Chamant succeeds Mondon (d. 31 December 1970) as Minister of Transport. Roger Frey becomes Minister of Administrative Reforms and is not replaced as Minister of Relations with Parliament.
 25 February 1971 – Pierre Messmer enters the ministry as Minister of Overseas Departments and Territories.

References

Social category, France, Commanding Heights, PBS official website

Further reading
Larry Collins and Dominique Lapierre, Is Paris Burning?, New York:  Pocket Books, 1965.

1915 births
2000 deaths
Politicians from Paris
Radical Party (France) politicians
Rally of the French People politicians
National Centre of Social Republicans politicians
Union for the New Republic politicians
Union of Democrats for the Republic politicians
Rally for the Republic politicians
Prime Ministers of France
Transport ministers of France
Government ministers of France
Presidents of the National Assembly (France)
Deputies of the 1st National Assembly of the French Fourth Republic
Deputies of the 2nd National Assembly of the French Fourth Republic
Deputies of the 3rd National Assembly of the French Fourth Republic
Deputies of the 1st National Assembly of the French Fifth Republic
Deputies of the 2nd National Assembly of the French Fifth Republic
Deputies of the 3rd National Assembly of the French Fifth Republic
Deputies of the 4th National Assembly of the French Fifth Republic
Deputies of the 5th National Assembly of the French Fifth Republic
Deputies of the 6th National Assembly of the French Fifth Republic
Deputies of the 7th National Assembly of the French Fifth Republic
Deputies of the 8th National Assembly of the French Fifth Republic
Deputies of the 9th National Assembly of the French Fifth Republic
Deputies of the 10th National Assembly of the French Fifth Republic
Mayors of Bordeaux
Candidates in the 1974 French presidential election
Lycée Lakanal alumni
Sciences Po alumni
French Resistance members
French people of the Algerian War
Sportspeople from Paris
France international rugby union players
Commanders of the Order of Isabella the Catholic
Sportspeople from Gironde
Rugby union wings

Jacques Chaban-Delmas


Wacław Tomasz Depo (Szydłowiec perto de Radom, Polônia, 27 de setembro de 1953) é arcebispo de Częstochowa (Czestochowa).

Depois de terminar o colegial, ele entrou no seminário em Sandomierz. Em 3 de junho de 1978 recebeu o Sacramento da Ordem de Piotr Gołębiowski, Administrador Apostólico da Diocese de Sandomierz. Depois de um curto vicariato, começou a estudar na Faculdade de Teologia da Universidade Católica de Lublin, em dezembro de 1980 recebeu seu mestrado e em 1984 recebeu seu doutorado. Em 1990 tornou-se regente do seminário de Sandomierz. Em 25 de março de 1992, encarnou na diocese de Radom, onde trabalhou como professor de dogmática no Colégio Teológico de Radom. Em 1999 Depo tornou-se presidente da Conferência de Reitores Alemães e de 2000 a 2006 foi professor assistente na Universidade Cardinal Stefan Wyszyński (UKSW) em Varsóvia.

Em 5 de agosto de 2006, o Papa Bento XVI o nomeou Bispo de Zamość-Lubaczów. O arcebispo de Przemyśl, Józef Michalik o consagrou bispo em 9 de setembro de 2006 na Catedral de Zamość-Lubaczów; Co-consagradores foram o Bispo de Radom, Zygmunt Zimowski, e seu antecessor Jan Śrutwa. Ele é o representante leigo da Conferência Episcopal Polonesa.

Em 29 de dezembro de 2011, Bento XVI o nomeou ao Arcebispo de Częstochowa. A posse ocorreu em 2 de fevereiro de 2012.

Ver também

Arcebispos católicos da Polônia

Wacław Tomasz Depo


Ulrike Rylance (* 1968 in Jena, DDR) ist eine deutsche Autorin.

Leben
Rylance studierte Anglistik und Germanistik in Leipzig und London. Während des Studiums arbeitete sie als Assistant Teacher in Wales und Manchester. Nach dem Studium blieb sie zunächst in London und arbeitete dort als Deutschlehrerin für Kinder und Erwachsene. Seit 2001 lebt sie als freischaffende Autorin in Seattle in den USA und hat zwei Töchter.

2010 erschien ihr erster Jugendroman, seither folgten weitere Kinder- und Jugendbücher sowie Unterhaltungsromane für Erwachsene. Sie schreibt auch unter den Pseudonymen Ulrike Herwig (Unterhaltungsromane für Erwachsene), Caro Martini (Frauenromane mit "Magic Twist") und Carly Wilson (High-School-Serie "Myriad High").

Werke
 Schiefer die Socken nie hingen, dtv, München, 2019. ISBN 978-3-423-28200-0
 Zweite Pause Zoff und Zucker, dtv, München, 2019. ISBN  978-3423-76259-5
 Das Leben ist manchmal woanders, dtv, München, 2018. ISBN  978-3423-26161-6
 Penny Pepper 7 – Diebesjagd in London, dtv, München, 2018. ISBN 978-3-423-76235-9
 Penny Pepper 6 – Auf Klassenfahrt, dtv, München, 2017. ISBN 978-3-423-76170-3
 Penny Pepper 5 – Spione am Strand, dtv, München, 2017. ISBN 978-3423761888
 Pias besonderes Talent, Ed. bi:libri, München, 2017. ISBN 978-3-19-999597-5
 Mein Mathe-Desaster oder der lange Weg zum ersten Kuss, dtv, München, 2016. ISBN 978-3-423-76140-6
 Penny Pepper 4 – Tatort Winterwald, dtv, München, 2016. ISBN 978-3-423-76162-8
 Penny Pepper 3 – Chaos in der Schule, dtv, München, 2015. ISBN 978-3-423-76129-1
 Penny Pepper 2 – Alarm auf der Achterbahn, dtv, München, 2015. ISBN 978-3423761178
 Penny Pepper 1 – Alles kein Problem, dtv, München, 2014. ISBN 978-3-423-76100-0
 Pia sucht eine Freundin, Ed. bi:libri, München, 2014. ISBN 978-3-19-509596-9
 Eiskaltes Herz, dtv, München, 2013. ISBN 978-3-423-71541-6
 Frieda aus der Flasche, illustriert von Regina Kehn, Jacoby & Stuart, Berlin, 2012. ISBN 978-3-941787-69-8
 Todesblüten, dtv, München, 2012. ISBN 978-3-423-78258-6
 Emma im Knopfland, illustriert von Silke Leffler, Jacoby & Stuart, Berlin, 2011. ISBN 978-3-941787-28-5
 Villa des Schweigens, dtv, München, 2011. ISBN 978-3-423-78249-4
 Pia kommt in die Schule, Ed. bi:libri, München, 2011. ISBN 978-3-19-079595-6
 Der Farbenverdreher, Ed. bi:libri, München, 2010. ISBN 978-3-938735-65-7
 Ein Date für vier: eine deutsch-englische Lovestory, dtv, München, 2010. ISBN 978-3-423-78247-0

Auszeichnungen
 2013: Prix Chronos für Frieda aus der Flasche
 2015: Hansjörg-Martin-Preis des Syndikats für Penny Pepper – Alles kein Problem

Weblinks

 Homepage von Ulrike Rylance
 http://www.jacobystuart.de/index.php?option=com_content&view=article&id=441
 http://www.dtv.de/autoren/ulrike_rylance_13717.html
 Rezension zu Villa des Schweigens auf KinderundJugendmedien.de

Einzelnachweise

Autor
Kinder- und Jugendliteratur
Deutscher
Geboren 1968
Frau

Ulrike Rylance


Luigi Tansillo (født 1510 i Venosa, død 1. december 1568 i Teano) var en italiensk digter.

Han var af adelig slægt og kom som ung ind i den æresgarde I continui, som tjente den spanske vicekonge Pedro Álvarez de Toledo og hans søn Don García, og i denne tjeneste foretog han mange rejser til lands og til vands. Efter Don Garcías død levede han i små kår, en tid som capitano i Gaeta. Tansillo er en af 16. århundredes bedste digtere, mere oprindelig end de fleste. Han har skrevet lyriske digte — sonetter og lignende —, som er mindre petrarkiserende end samtidens øvrige, hyrdedigte, fulde af komplimenter til hans fornemme beskyttere, burleske capitoli, i Bernis stil, et temmelig slibrigt poem med titlen Il Vendemmiatore (1532) og til bod for dette et gudeligt epos Le lagrime di San Pietro (ligeledes begyndt i hans ungdom, ufuldendt ved hans død). Skønt sidstnævnte er noget ensformigt og ikke videre begejstret, fik det stort ry og udkom ofte, længe dog i en af inkvisitionen beskåret udgave, først 1606 som det forelå fra Tansillos hånd; det blev oversat på spansk og fransk og efterlignet af Malherbe i Les larmes de Saint Pierre. Gode udgaver af Tansillos poetiske arbejder (forskellige samlinger) blev besørgede af Scipione Volpicella (1870), Francesco Fiorentino (1882) og Francesco Flamini (1893), alle i Neapel.

Kilder

Digtere fra Italien
Italienere i 1500-tallet

Luigi Tansillo


Spirotaenia condensata, vrsta slatkovodne parožine iz porodice Mesotaeniaceae. Pronađena je po svim kontinentima osim Afrike.

Sinonimi
 Entospira condensata (Brébisson) Kuntze 1898

Izvori

Vanjske poveznice

Zygnematales

Spirotaenia condensata


المِدْفَأَة هي أي جهاز يستخدم في التدفئة في الأجواء الباردة.

قديماً كانت المدفأة البدائية عبارة عن مجموعة من الحطب وأغصان الأشجار يشعل فيها الإنسان النار، وقد استخدم الإنسان البدائي الذي عاش في الكهوف والغابات هذا النوع من المدافئ.

ثم تطورت بعدما تمكن الإنسان من بناء المنازل لتصبح عبارة عن فجوة في الحائط مصنوعه من الطوب الحراري، يضع فيها الإنسان الفحم والحطب وأغصان الأشجار ويقوم بإشعالها.

و بعد اكتشاف الكهرباء تم اختراع المدفأة الكهربائية المعروفة في عالمنا اليوم.

كما تعد المدفأة أحد خواص بعض أنواع مكيفات الهواء، ولا يقتصر وجود المدفأة اليوم على المنازل فقط، حيث أنها توجد في بعض السيارات كأحد خواص مكيفات هوائها. ويوجد هناك أنواع أخرى مثل المدافئ التي تعمل على الوقود مثل الكيروسين والديزل، ولكنها أكثر خطراً على البيئة. وبعد التطور أصبحنا نستخدم المكيف وهو آلة ملتصقة بالحائط نستطيع من خلالها أن نحصل على جو بارد في الصيف وجو حار في الشتاء.

انظر أيضًا
 مدفأة كيروسين (مدفأة نفطية)
 مدفأة زيتية
 مدفأة غازية
 تسخين كهربائي (مدفأة كهربائية)
 موقد
 موقد حطب
 موقد فرانكلين
 خزان حرارة
 خزان حراري لا نهائي

المراجع

تدفئة
حرارة

مدفأة


Jarlsberg ( , ) is a mild cheese made from cow's milk, with large, regular eyes, originating from Jarlsberg, Norway. It is produced in Norway, as well as in Ireland and the US state of Ohio, licensed from Norwegian dairy producers. It is classified as a Swiss-type cheese.

Description
Jarlsberg cheese has a yellow wax rind (outer layer) and a semi-firm yellow interior. It is a mild, buttery cheese. The flavour has been described as "clean and rich, with a slightly sweet and nutty flavour".  It is an all-purpose cheese, used for both cooking and eating as a snack. It has a characteristic smooth, shiny-yellow body, and a creamy, supple texture. It is aged a minimum of three months and is distinguished by medium to large holes. Some variations are aged a minimum of 9, 12 or 15 months. It is usually produced in  wheels with an approximate diameter of  and a height of . The characteristic holes or "eyes" are the result of the action of the bacteria Propionibacterium freudenreichii which naturally occurs in milk and is added back into the cheese during production according to a closely guarded secret formula.

History
The history of this cheese can be traced back to the middle 1850s. Anders Larsen Bakke (1815–1899), a farmer and pioneer in Norway's dairy industry, produced cheese in the village of Våle in what was then the county of Jarlsberg and Larviks Amt (now Vestfold),  south of Oslo. The cheese shares similarities with Emmental, introduced to Vestfold by Swiss cheese makers during the 1830s. The cheese was first noted in the annual county report of Jarlsberg and Larviks Amt in 1855. After several years of popularity marked by a large volume of production Jarlsberg disappeared from the market.

Modern Jarlsberg cheese was developed in 1956 by Ole Martin Ystgaard of the Dairy Institute at the Agricultural University of Norway. Ystgaard's interest was sparked by the thesis of a dairy sciences student, Per Sakshaug, on the cheese historically made in Vestfold. It was named for a Norwegian nobleman, Count Wedel Jarlsberg, who owned land near Oslo in an area where an earlier version of the cheese was produced in the early 1800s, or for the eponymous county. The recipe was developed from formulae originating with Swiss cheesemakers who moved to Norway at that time.

Production and distribution

"Jarlsberg" is a trademark first registered by Tine SA in 1972, and the exact nature and formula for the process of making Jarlsberg cheese is a trade secret.  The largest producer of Jarlsberg cheese is Tine SA. Tine is the largest Norwegian dairy product cooperative. Jarlsberg cheese accounts for 80% of Tine's total exports. Tine's United States subsidiary, Norseland, has sold 150 million  wheels of Jarlsberg cheese in the U.S. .

Jarlsberg cheese was introduced in the United States in 1964. Imports to the U.S. in 1965 were . Since 1979 imports to the U.S. have been limited to . Jarlsberg is the most popular imported cheese in the U.S. ,  of Jarlsberg cheese was made in the U.S. in Ohio. It is also produced in Ireland by Dairygold.

Annual sales of Jarlsberg cheese in the United Kingdom are £6.9m . Jarlsberg cheese is also popular in Australia.
Jarlsberg is used as the topping of the Grandiosa, the best-selling frozen pizza in Norway.

See also
 Emmental cheese
 Leerdammer
 List of cheeses
 Maasdam cheese
 Norvegia

References

External links
 Jarlsberg.com site of brand owner.

Cow's-milk cheeses
Norwegian cheeses
Cheese with eyes

Jarlsberg cheese


Hereditary coproporphyria (HCP) is a disorder of heme biosynthesis, classified as an acute hepatic porphyria.  HCP is caused by a deficiency of the enzyme coproporphyrinogen oxidase, coded for by the CPOX gene, and is inherited in an autosomal dominant fashion, although homozygous individuals have been identified.  Unlike acute intermittent porphyria, individuals with HCP can present with cutaneous findings similar to those found in porphyria cutanea tarda in addition to the acute attacks of abdominal pain, vomiting and neurological dysfunction characteristic of acute porphyrias. Like other porphyrias, attacks of HCP can be induced by certain drugs, environmental stressors or diet changes.  Biochemical and molecular testing can be used to narrow down the diagnosis of a porphyria and identify the specific genetic defect.  Overall, porphyrias are rare diseases.  The combined incidence for all forms of the disease has been estimated at 1:20,000.  The exact incidence of HCP is difficult to determine, due to its reduced penetrance.

Signs and symptoms
Clinically, patients affected with HCP present similarly to those with other acute porphyrias, such as acute intermittent porphyria (AIP) and variegate porphyria (VP).  Patients with HCP and VP can present with symptoms shared between the acute and cutaneous porphyrias.  This includes the acute attacks of abdominal pain, nausea, vomiting, diarrhea, tachycardia, hypertension and seizures, as well as the cutaneous findings seen in porphyria cutanea tarda (PCT), namely increased skin fragility, bullous lesions after exposure to sunlight and increased scarring.

Individuals with HCP may be asymptomatic in the absence of triggering factors.  Common triggers include certain drugs, alcohol, hormonal changes, and dietary changes. Sunlight and other ultraviolet light can trigger the skin manifestations.  Homozygous individuals for CPOX mutations can present with these findings at an earlier age than heterozygotes.

Genetics
HCP is caused by mutations in CPOX, which codes for the enzyme coproporphyrinogen oxidase.  This enzyme is responsible for the sixth step in the heme biosynthetic pathway, converting coproporphyrinogen III to protoporphyrinogen IX.   CPOX is located at 3q11.2-q12.1, has 6 introns and 7 exons and produces an mRNA strand that is 2675 bases in length.  It is inherited in an autosomal dominant fashion, meaning that a deficiency of 50% of the normal enzyme activity is enough to cause symptoms.  As reproductive fitness is not impacted, homozygous affected individuals have been reported.  Along with other acute porphyrias HCP demonstrates reduced penetrance, meaning not all individuals who carry a disease-causing mutation will express symptoms.

Individuals who are homozygous for a specific mutation (K404E) or compound heterozygous with a null allele in CPOX have a more severe erythropoietic porphyria, harderoporphyria, characterized by neonatal jaundice, hyperbilirubinemia, hepatosplenomegaly and skin lesions upon exposure to ultraviolet light.  HCP is a rare disease, but the exact incidence is difficult to determine due to the reduced penetrance of the acute porphyrias.  Overall, the incidence of all porphyrias is estimated at 1:20,000 in the United States.  The incidence of harderoporphyria is even lower, with less than 10 cases reported worldwide.

Diagnosis
The diagnosis of any porphyria is often delayed due to the rarity of the disease as well as the varied and non-specific findings that patients present with.  Bedside measurement of urine porphobilinogen is recommended as a screening test for patients suspected of having an acute porphyria.  Elevated porphobilinogen is indicative of an acute porphyria, and additional testing can be done to narrow down the specific type.

The identification of a specific porphyria is based on the results of laboratory findings, including blood, urine and stool tests.  HCP can be distinguished from most other acute porphyrias by the cutaneous findings.  VP presents similarly, but can be distinguished based on urine and stool porphyrin analysis, typically done using high performance liquid chromatography with fluorescence detection.  The results of biochemical testing for porphyrias are most informative when samples are collected during an acute attack.  Typically, the distinguishing metabolite for HCP and VP is the presence of protoporphyrin in the plasma and feces of individuals affected with VP.

Elevated coproporphyrin is a common finding in urine, known as coproporphyrinuria as it is the predominant porphyrin species in urine.  This is a non-specific finding that is not necessarily due to an acute porphyria.  Coproporphyrinuria can be caused by other stressors to the heme biosynthetic pathway, such as liver disease, lead poisoning and certain bone marrow disorders.

Treatment
There is no cure for HCP caused by the deficient activity of coproporphyrinogen oxidase.  Treatment of the acute symptoms of HCP is the same as for other acute porphyrias.  Intravenous hemin (as heme arginate or hematin) is the recommended therapy for acute attacks.  Acute attacks can be severe enough to cause death if not treated quickly and correctly.  Hospitalization is typically required for administration of hemin, and appropriate drug selection is key to avoid exacerbating symptoms with drugs that interact poorly with porphyrias.  Proper drug selection is most difficult when it comes to treatment of the seizures that can accompany HCP, as most anti-seizure medications can make the symptoms worse.  Gabapentin and levetiracetam are two anti-seizure drugs that are thought to be safe.

In patients where management of symptoms is difficult even with hemin, liver transplant is an option before the symptoms have progressed to advanced paralysis. Combined liver and kidney transplants are sometimes undertaken in people with kidney failure.

Long term treatment of acute porphyrias is centered on the avoidance of acute attacks by eliminating precipitating factors, such as drugs, dietary changes, and infections.  Females often have attacks coincident with their menstrual cycle, which can be managed effectively with hormonal birth control.  Because of the reduced penetrance of HCP, family members of a patient may carry the same mutation without ever presenting with symptoms.  Molecular analysis of CPOX is the best way to identify these patients, as they will not express a biochemical phenotype on laboratory testing unless they are symptomatic.  Identification of asymptomatic patients allows them to adjust their lifestyle to avoid common triggering factors.

References

External links

Porphyrias
Skin conditions resulting from errors in metabolism

Hereditary coproporphyria


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